Complex 1 protein (LYR family); Proteins in this family include an accessory subunit of the higher eukaryotic NADH dehydrogenase complex. In Saccharomyces cerevisiae, the Isd11 protein has been shown to play a role in Fe/S cluster biogenesis in mitochondria. We have named this family LYR after a highly conserved tripeptide motif close to the N-terminus of these proteins.

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gi 10092689   20 QQVLLLYRRILQTIRQVPNDSDRKYLKDWAREEFRRNKSATEEDTIRMMITQGNMQLKE 78
Cdd:pfam05347  1 RQVLSLYRALLRAARKFPDYNFREYFRRRIRDEFRKNKDLTDPEKIEKLLKEGKEELEE 59

LYR (leucine-tyrosine-arginine) motif found in Complex1_LYR-like superfamily; The Complex1_LYR-like superfamily consists of proteins of diverse functions that are exclusively found in eukaryotes and contain the conserved tripeptide 'LYR' close to the N-terminus. The human genome has at least ten LYR proteins that were predominantly identified as mitochondrial proteins. Some family members were also found in the cytosol or nucleus. LYR motif-containing protein 4 (LYRM4) represents the only LYR protein that is directly involved in the first steps of Fe-S cluster generation. Other LYR proteins have been identified as accessory subunits or assembly factors of mitochondrial OXPHOS (oxidative phosphorylation) complexes I, II, III and V, and they play specific roles in acetate metabolism.

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gi 10092689 22 VLLLYRRILQTIRQVPNDSDRKYLKDWAREEFRRNKSATEEDTIRMMITQGNMQLKE 78
Cdd:cd20251  1 VLKLYRDLLRLARKGLPSRKRDALRQRIREEFRKNKNETDPEKIEELLAEARRGLKN 57

LYR (leucine-tyrosine-arginine) motif found in LYR motif-containing protein 1 (LYRM1) and similar proteins; LYR motif-containing protein 1 (LYRM1) may promote cell proliferation and inhibition of apoptosis of preadipocytes. Overexpression of the human LYRM1 causes mitochondrial dysfunction and induces insulin resistance in 3T3-L1 adipocytes. LYRM1 belongs to the Complex1_LYR-like superfamily that consists of proteins of diverse functions that are exclusively found in eukaryotes and contain the conserved tripeptide 'LYR' close to the N-terminus.

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gi 10092689 21 QVLLLYRRILQTIRQVPNDSD--RKYLKDWAREEFRRNKSATEEDTIRMMITQGNMQL 76
Cdd:cd20261  1 EVLSLYRRILRLARTWPADTEeeREYILNEARSLFRKNKNLTDPEEIEEKIQEAESRL 58

Complex1_LYR-like; This is a family of proteins carrying the LYR motif of family Complex1_LYR, pfam05347, likely to be involved in Fe-S cluster biogenesis in mitochondria.

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gi 10092689   22 VLLLYRRILQTIRQVPNDSdRKYLKDWAREEFRRNKSATEEDTIRMMITQGNMQLKELEK 81
Cdd:pfam13233  1 VLSLYRALLRELRELPPNG-RSYGRQYLRAEFRRHKHLTDPEEIAEFLKSWREYKELLER 59

LYR (leucine-tyrosine-arginine) motif found in electron transfer flavoprotein regulatory factor 1 (ETFRF1) and similar proteins; ETFRF1, also called LYR motif-containing protein 5 (LYRM5), or Ghiso (growth-factor inducible soluble) factor, acts as a regulator of the electron transfer flavoprotein by promoting the removal of flavin from the ETF holoenzyme (composed of ETFA and ETFB). It belongs to the Complex1_LYR-like superfamily that consists of proteins of diverse functions that are exclusively found in eukaryotes and contain the conserved tripeptide 'LYR' close to the N-terminus.

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gi 10092689 19 RQQVLLLYRRILQTIRQVPNDSDrkYLKDWAREEFRRNKSATEEDTIRMMITQGNMQLKELE 80
Cdd:cd20265  1 RSRVRQLYKRLLYLGRDYPGGLD--YFRDRLKRAFMKNKDLTDPEEIKKAIARGEYVIKELE 60

LYR (leucine-tyrosine-arginine) motif found in LYR motif-containing protein 7 (LYRM7) and similar proteins; LYRM7 is an assembly factor required for Rieske iron sulfur (Fe-S) protein UQCRFS1 incorporation into the cytochrome b-c1 (CIII) complex. It functions as a chaperone, binding to this subunit within the mitochondrial matrix and stabilizing it prior to its translocation and insertion into the late CIII dimeric intermediate within the mitochondrial inner membrane. LYRM7 mutations cause a multifocal cavitating leukoencephalopathy with a distinct and recognizable magnetic resonance imaging (MRI) pattern. LYRM7 belongs to the Complex1_LYR-like superfamily that consists of proteins of diverse functions that are exclusively found in eukaryotes and contain the conserved tripeptide 'LYR' close to the N-terminus.

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gi 10092689 22 VLLLYRRILQTIRQV-PNDSD-RKYLKDWAREEFRRNKSATEEDTIRMMITQGNMQLKELEKTLALAK 87
Cdd:cd20267  1 VLSAYRELLRARRWVfKGDTPaLKAARQKIREEFEKNRNETDPEKIAELLKEAREAAEFLRKNVVQAV 68

LYR (leucine-tyrosine-arginine) motif found in NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 (NDUFA6) and similar proteins; NDUFA6, also called LYR motif-containing protein 6 (LYRM6), or Complex I-B14 (CI-B14), or NADH-ubiquinone oxidoreductase B14 subunit, is an accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), and is believed to be not involved in catalysis. In general, accessory subunits are integral for assembly and function of Complex I, which functions in the transfer of electrons from NADH to the respiratory chain. NDUFA6 belongs to the Complex1_LYR-like superfamily that consists of proteins of diverse functions that are exclusively found in eukaryotes and contain the conserved tripeptide 'LYR' close to the N-terminus.

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gi 10092689 19 RQQVLLLYRRILqtiRQVPN-------DSDRKYLKDWAREEFRRNKSATEEDTIRMMITQGNMQLKEL 79
Cdd:cd20266  5 RRRVLQLYRAWY---RSIPTiveiynlDITVSQLRSKIREEFRKNAHVTDPRVIDMLVIKGQMELQET 69

LYR (leucine-tyrosine-arginine) motif found in mitochondrial succinate dehydrogenase assembly factor 1 (SDHAF1) and similar proteins; SDHAF1, also called SDH assembly factor 1, or LYR motif-containing protein 8 (LYRM8), is a LYR complex-II specific assembly factor that plays an essential role in the assembly of succinate dehydrogenase (SDH), an enzyme complex (also referred to as respiratory complex II) that is a component of both the tricarboxylic acid (TCA) cycle and the mitochondrial electron transport chain, and which couples the oxidation of succinate to fumarate with the reduction of ubiquinone (coenzyme Q) to ubiquinol. It promotes maturation of the iron-sulfur protein subunit SDHB of the SDH catalytic dimer, protecting it from the deleterious effects of oxidants. SDHAF1 may act together with SDHAF3. It is mutated in SDH-defective infantile leukoencephalopathy. SDHAF1 belongs to the Complex1_LYR-like superfamily that consists of proteins of diverse functions that are exclusively found in eukaryotes and contain the conserved tripeptide 'LYR' close to the N-terminus.

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gi 10092689 19 RQQVLLLYRRILQTIRQVPnDSDRKYLKDWAREEFRRNKSATEED--TIRMMITQGNMQLK 77
Cdd:cd20268  4 QKQVLSLYRSFLRAARSKP-GENRPGFRAYIRDEFRKNASIPRKDflTIEHLLRRGQRQLE 63